BACKGROUND: Because of the rarity of right aortic arch coarctation there are few reports of large groups of patients. OBJECTIVE: To characterize the frequency and type of right aortic arch coarctation in a large group of pediatric patients. MATERIALS AND METHODS: From June 1997 through May 2007, 11,276 consecutive children with congenital heart disease underwent multidetector CT (MDCT), MRI or angiocardiography examination. All children with a right aortic arch or coarctation were reviewed. RESULTS: Right aortic arch coarctation was found in 11 children representing 0.1% of the total group of 11,276 children, 1.7% of 658 children with native coarctations and 2.3% of 473 children with a right aortic arch. Among the 11 patients, 6 had long-segment narrowing and 7 had an aberrant left subclavian artery. CONCLUSION: MDCT, MRI and angiocardiography are reliable imaging techniques for the diagnosis of right aortic arch and coarctation. Our findings showed that the pattern of right aortic arch coarctation was different from that of left aortic arch coarctation, suggesting that they are different etiological entities. The pivotal role possibly played by flow dynamics in the development of right aortic arch coarctation is discussed.